| Summary: | A Thesis submitted to The University of Arizona College of Medicine - Phoenix in partial fulfillment of the requirements for the Degree of Doctor of Medicine. === 3
Abstract
Background: Hyperbilirubinemia in pediatric hematopoietic stem cell transplant (HSCT) patients presents a diagnostic and therapeutic dilemma. This study was designed to determine the frequency of known causes of hyperbilirubinemia, correlate its effect on survival, review the effects of gallbladder procedural interventions on outcomes, and to define parameters to guide patient care.
Methods: 60 Pediatric HSCT patients transplanted between August 2003 and March 2010 were stratified according to bilirubin levels, presence of Veno-occlusive Disease (VOD), also known as Sinusoidal Occlusive Syndrome (SOS), gallbladder involvement, and surgical intervention. Subgroups were analyzed for peak liver enzyme levels, Intensive care unit (PICU)/hospital length of stay (LOS), and mortality.
Results: Fifty-five per cent of patients developed hyperbilirubinemia. Survival was 55% in that group versus 85% in the normo-bilirubin patients. 79% of the hyperbilirubinemia patients exhibited gallbladder involvement and 31% of those underwent surgical intervention. Survival was 60% in the patients with gallbladder involvement without SOS who underwent surgery versus 50% in the non-surgical. In patients with both SOS and gallbladder involvement, survival was 33% in the intervention group versus 50% in the non-intervention group. Overall survival in the SOS alone, SOS with gallbladder involvement, and gallbladder involvement only patients was 50%,
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44%, and 53% respectively. Hepatic enzymes were increased in patients who received intervention compared to the non-intervention groups, but no individual lab or combination of labs predicted intervention or mortality. Intervention patients spent more days in the hospital post bilirubin peak.
Conclusions: Pediatric HSCT patients with elevated bilirubin levels and SOS are at increased risk for gallbladder complications and death. Patients receiving gallbladder procedures are more likely to require longer hospital stays than patients who do not. Surgical intervention may decrease mortality in patients with gallbladder involvement that do not have SOS, but no conclusion regarding a positive impact of gallbladder intervention on patients with coexisting SOS can be made on other patients based on this data set.
Keywords: Bilirubin, Hyperbilirubinemia, Hematopoietic stem cell transplant, Cholecystectomy, Cholecystostomy, Veno-occlusive disease, Sinusoidal obstructive syndrome
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